【摘要】 目的:评估多发性骨髓瘤(multiple myeloma,MM)合并肾淀粉样变性的临床特征、疗效及预后。方法:回顾性分析我院2011年11月-2015年07月期间新发的22例MM合并肾淀粉样变性患者的临床资料。结果:22例患者ISS分期Ⅱ期占77.2%(17/22),Ⅲ期为22.8%(5/22)。肾功能损害占36.4%(8/22),贫血占40.9%(9/22),血清白蛋白<35 g/L占86.4%(19/22),尿蛋白阳性100%(22/22)。22例中完全缓解(CR)13.6%(3/22),非常好的部分缓解(VGPR)4.5%(1/22),部分缓解(PR)22.8%(5/22),疾病稳定(SD)45.5%(10/22),疾病进展(PD)13.6%(3/22),其中9例治疗有效患者,肾脏淀粉样变性有3例改善,4例稳定,2例进展。可随访17例患者中,7例死亡,10例存活,平均随访11(1-37)个月,中位总体生存19(95%CI 9.2-28.8)个月。结论:MM合并肾淀粉样变性临床上少见、难治、预后差。MM出现大量蛋白尿特别是合并肾病综合征时,无论是否有肾功能损害,均需考虑合并有肾淀粉样变性。以硼替佐米为基础的化疗可能提高患者疗效。更多还原

【Abstract】 Objective:To evaluate the clinical characteristics of multiple myeloma(MM) combined with renal amyloidosis and its curative efficacy and prognosis.Methods:The clinical data of 22 cases of newly diagnosed multiple myeloma combined with renal amyloidosis treated in our hospital from November 2011 to July 2015 were analyzed retrospectively.Results:According to Intenational Staging System(ISS),among above- menthioned 22 patients the ISS Ⅱ accounted for 77.2%(17/22),ISS Ⅲ accounted for 22.8%(5/22).The patients with renal impairment accounted for 36.4%(8/22),with anemia 40.9%(9/22),with serum album < 35 g/L 86.4%(19/22),with urinary protein positive 100%(22/22).The evaluation of the curative efficacy of the 22 cases was as follows:CR 13.6%(3 /22);VGPR4.5%(1/22);PR 22.8%(5/22);SD 45.5%(10/22);PD 13.6%(3/22).Out of 9 patients with effective treatment,3 cases(3/9 33.3%) achieved "improved" in renal amyloidosis,4 cases(4/9,44.5%) achieved stable in renal amyloidosis,2 cases(2/9 2%) achieved "worsened" in renal amyloidosis.Among 17 cases who were followed up,7 cases died,10 cases survived,the average duration of follow-up for these cases was 11(1-37) months,the median overall survival(OS) time was 19(95%CI 9.2-28.8) months.Conclusion:MM with renal amyloidosis is rare,refractory and has a poor prognosis.Whether there is impairment of kidney function or not,renal amyloidosis shall be taken into consideration if the MM patients got massive proteinuria especially nephritic syndrome.Bortezomib may improve the curative efficacy.更多还原